Kawasaki disease is now being recognized as the leading cause of acquired heart disease in children in North America and Japan. This review discusses the recent developments and controversies in Kawasaki disease. Increasing evidence has supported an infectious etiology for Kawasaki disease; however, the debate continues as to whether the inflammatory response results from a conventional antigen or a superantigen. Recent immunohistochemistry findings suggest many vascular growth factors then play a role in the formation of the coronary artery lesions. Many studies have focused on the identification and therapy for patients resistant to conventional therapy, as well as the long-term prognosis of Kawasaki disease survivors. The recent advances in Kawasaki disease are helping to provide some clues in the etiology, pathogenesis and therapy for Kawasaki disease.